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British Journal of Ophthalmology, 1980, 64, 2-6
A new treatment for congenital nystagmus
R. V. ABADI, D. CARDEN, AND J. SIMPSON
From the University of Manchester Institute of Science and Technology, Visual Sciences Laboratory,
Department of Ophthalmic Optics, PO Box 88, Manchester M60 IQD
SUMMARY Congenital nystagmus is a disorder of eye movement with an associated reduction in
visual acuity. The latter is mainly due to the intensity of the nystagmus (amplitude times frequency)
allowing the object of regard to spend only a short time on the fovea. Training patients by an
auditory feedback technique to control the nystagmus enables visual sensitivity to improve. This
method is compared with present alternatives, and further studies are suggested.
major visual problem, how can the clinician remedy
the situation? In the past a number of techniques,
both optical and surgical, have been attempted,
though none seem able to provide a suitable method
for the diversity of cases encountered. Moreover,
the specific nature of these therapies do not make
them readily modifiable (see Discussion).
It is the aim, therefore, of this paper to report a
new method which should provide an improvement
in visual sensitivity for most individuals with CN.
Simply, we proposed to let the subjects of our
study hear their own eye movements and thereby
attempt to use this feedback signal to control their
nystagmus.
Estimations of the incidence of congenital nystagmus
(CN) in the general population vary enormously.
The data of Hemmes (1926), 1 in 5000, Anderson
(1953), 1 in 350, and Norn (1964), 1 in 20 000,
provide a surprisingly large range, which most
probably reflects not only the researchers' differing
definitions of the condition but also the manner in
which they collected the data. Perhaps a more
clinically pertinent survey was that made by Fine
(1968). She found that in England and Wales
nystagmus was the fourth largest (11-2-14%)
cause of partial sight.
In this study CN is defined as involuntary ocular
oscillations occurring at birth or soon after (3
months), owing its origin to genetic, intrauterine,
or birth influences. Characteristically, the dominant
eye movements are bilateral and horizontal, although
on occasion a vertical nystagmus, which usually
signifies an acquired disorder, has been reported as
being inherited (Forsythe, 1955; Marmor, 1973).
The intensity (amplitude x frequency) of the
nystagmus may vary enormously from individual
to individual, and it may alter for each direction of
gaze (Dell'Osso, 1973; Abadi and Sandikcioglu,
1974). Visual sensitivity for both pattern and
movement detection is reduced because of these
eye movements (Abadi, 1974a; Abadi and Sandikcioglu, 1975). This is understandable, since the
object of regard spends little time in the foveal
area, and the blur caused by the image movement,
often in excess of 50°/s, is considerable (Abadi and
Sandikcioglu, 1974; Dell'Osso and Daroff, 1975).
In congenital nystagmus no oscillopsia (apparent
movement of the environment) is noticed.
Accepting that the congenital nystagmat has a
Methods
To be able to understand any eye movement disorder
and monitor its improvement after therapy both
the motor and sensory responses need to be examined. These include nystagmus intensity and the
visual sensitivity.
RECORDING THE EYE MOVEMENTS
A modified photoelectric method was employed to
monitor the nystagmus (Stark et al., 1962). Infrared
sources (TIL 24) and receivers (LS 614) were used to
detect changes in limbal reflection due to the
involuntary horizontal nystagmus (Fig. 1). These
devices were mounted on a small printed circuit
board and attached to the spectacles of the observer
(Fig. 2). The reflected infrared light is detected by
the receiver and fed into a preamplifier, the resultant
amplified signal being then used to control the
frequency of a voltage-controlled oscillator operating
in the audiorange of 200-2000 Hz. The output of
this oscillator, after amplification, is used to operate
Correspondence to Dr R. V. Abadi.
2
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A new treatment for congenital nystagmus
3
Fig. 1 The infrared source
and photocell receiver
arrangement for recording eye
movements
I
' \I/
I
Illuminoted areas
ot eye
a small loudspeaker. Thus the subject can hear a
tone, the frequency of which is altered by the eye
movements.
Using this feedback device we have attempted to
control the degree of nystagmus by asking the
subjects to try to keep the tone constant. In order
to assess the degree of success of this technique we
monitored the intensity and velocity of the nystagmus, before, during, and after the course of training.
MEASURING CONTRAST THRESHOLDS
At all stages of training, psychophysical contrast
sensitivity functions were measured to give an
indication of improved visual sensitivity. Both
vertical and horizontal sinusoidal grating patterns
were generated on the face of a Telequipment D61
oscilloscope (P31 phosphor), and each observer
was asked to find his own contrast threshold at
different spatial frequencies by adjusting a potentiometer which altered the contrast of the patterns
(Schade, 1956; Campbell and Green, 1965; Abadi,
1974b; Arden, 1978). The contrast of the grating
pattern is defined as the ratio of the difference of the
maximum and minimum luminance over their sum.
Contrast sensitivity is the inverse of contrast
threshold, and spatial frequency is an equivalent
measure of bar size in a grating pattern; that is, it
is the number of alternating pairs of dark and
Fig. 2 Subject using the eye movement recording
spectacles and listening for a change in tone from
the speaker
bright bands of the pattern subtended in degrees of
visual angle at the observer's eye. The mean screen
liminance was constant at 7 cd/M2, subtending an
angle of 80 when the viewing distance was 57 cm.
Monocular viewing of the circular display was used
throughout, and all subjects were fully corrected for
any refractive errors.
SUBJECTS
Although we have now 'trained' a number of
subjects to use the equipment, this paper will detail
the typically favourable results achieved by describing the data of one such observer, a 22-year-old
male. After trial runs in the laboratory he took the
feedback equipment home and over a period of 24
weeks practised keeping the tone constant for 5-10
minutes each morning and evening.
Results
Contrast sensitivity functions for prefeedback and
postfeedback are illustrated in Figs. 3a and b. The
visual sensitivity to horizontal patterns is seen to
be greater than that for vertical ones and confirms
the earlier studies of Abadi (1974a) and Abadi and
Sandikcioglu (1975). This effect is caused by the
horizontal motion of the nystagmus and is not due
to any uncorrected refractive error or meridional
amblyopia from an uncorrected astigmatism acquired early in life. Indeed it has recently been
demonstrated by Abadi and King-Smith (in press)
that if the eye movements are eliminated the orientational sensitivity bias is still present, suggesting
that the oculomotor disturbance may be responsible
for depriving the cortical orientational detectors of
a normal visual input and thus giving rise to a
meridional amblyopia.
After feedback training visual sensitivity for
both meridians is shown to have improved. Moreover the intensity of the nystagmus has been reduced
from an initial level of 3.590 ±0.360/4.75 Hz
+0t33 Hz to 2 640 ±0 290/3 88 Hz ±0-28 Hz
(Fig. 3, insets). In terms of the velocity of the image
moving in the back of the retina this would consti-
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R. V. Abadi, D. Carden, and J. Simpson
4
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250
200
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100
100
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50
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3 4 5 6
2
Spatial frequency (cycles deg-1)
3a
2
3 4 5 6
0-7 1-0
Spatial f requencv (cycles deg 1 )
3b
Fig. 3 Spatial contrast sensitivity functions for an observer with congenital nystagmus before (a) and after (b)
auditory feedback training. The filled and empty symbols refer to thresholds (mean of 20) in response to
horizontally and vertically orientated patterns respectively. Insets illustrate the form and intensity of the nystagmus
tute an approximate reduction from a value of
54°/s to 32°/s. Consequently the image, which is
now less blurred, also spends more time on the
fovea. Fig. 4 shows the postfeedback to prefeedback
contrast sensitivity ratios for viewing a horizontal
grating pattern (top) and a vertical pattern (bottom),
indicating sensitivity improvements of 0-17 (x 1 5)
and 030 (x 2 0) log units respectively. This change
was maintained after training had ceased.
In our studies the subjects were adults and
showed feedback changes of between 013 to 032
log units.
Discussion
Using a biofeedback technique we are able to
report that after training it is possible to improve
the visual sensitivity and hence the visual resolution
of adults with CN. Indeed this 'learned self-control'
is also illustrated by a reduction in both the amplitude and frequency of the nystagmus (Fig. 3,
insets). Of course, this does not mean that the
familiar methods of management of nystagmus,
namely, optical and surgical, will be replaced.
Rather the training routine could augment these
more traditional and specific therapies. Consequently it would not be out of order briefly to
consider them here and reflect on their limitations.
TRADITIONAL MANAGEMENT OF NYSTAGMUS
Optical treatment. Contact lenses have been used
as an optical aid. Their advantages for patients
with albinism or aniridia can be immense (Enoch
and Hudson, 1968), because the incident light
entering the eye can be more easily controlled.
Claims have also been made that the superiority of
the optical system of a contact lens over a spectacle
lens can be an important factor in improving
vision (Grosvenor, 1963). From an optical point
of view the contact lens correction should provide
fewer distortions and aberrations, and the prismatic
displacements which occur with spectacle lenses
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5
A new treatment for congenital nystagmus
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eyes to coincide with this position. Two types of
operations may be carried out. The first entails the
relocation of the eyes by operating on all 4 horizontal recti (Anderson, 1953; Kestenbaum, 1953).
The second, a more drastic approach, entails
immobilisation, either by fixing the ocular muscles
to the periostium (Friede, 1956) or by the less
extreme retroequatorial recession method (Bietti,
1956; Crone, 1971).
AUDITORY FEEDBACK
The techniques just described are specialised solutions to particular aspects of CN and do not offer
a general solution that is both practical and simple.
For example, prism therapy relies on the existence of
2- 5
a marked neutral point. Alternatively, by using
base in prisms it can present after short periods of
time symptoms typical of eye strain (Wybar,
2-( ) AA
--1974). Surgery is primarily attempted to counter
torticollis and is complicated if a strabismus is also
present. Moreover it is appropriate only if the
nystagmus is jerky (both fast and slow phases). On
1.'5
the other hand, biofeedback offers the subject a
self-trainer to be used either by itself or in conjuncotion with other optical or surgical methods.
1 0 l
Although the reported improvements in visual
/
sensitivity never reached levels greater than 0-32 log
A
units, it may be argued that the visual system is
5,
,
far from plastic in adults (Barlow, 1975). A study
,
, ,
is therefore now in progress to find the effects of
0-7 1-O
2
3 4 5 6
the same training schedule on young children where
the visual system is known to be more adaptable.
_-
Spatial frequency (cycles deg-1)
Fig. 4 Contrast sensitivity ratios comparing
prefeedilack with postfeedback data. The upper graph
illustratoes the postfeedback/prefeedback ratio for
horizonttal stimuli, and the lower graph shows the
equivalent ratio for vertical stimuli
As a further use, this auditory signal technique
can be extended to detecting and correcting other
oculomotor deviations, for example, squints, and
be used to train accurate fixation in individuals with
amblyopia.
We thank Miss Wiggie for her comments and drawings and
Miss Price for typing the manuscript. R. V. Abadi received
because of the moving eye are much reduced with financial
support from the British Optical Association and
the contact lens systems.
the Visual Research Trust.
Quite often the amplitude and frequency of CN
varies with positions of gaze, and in one direction, References
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A new treatment for congenital
nystagmus.
R V Abadi, D Carden and J Simpson
Br J Ophthalmol 1980 64: 2-6
doi: 10.1136/bjo.64.1.2
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